“Verteporfin exhibits anti-proliferative activity in embryonal and alveolar rhabdomyosarcoma cell lines”

Publication date: Available online 6 September 2019Source: Chemico-Biological InteractionsAuthor(s): Luca Sanna, Roberta Piredda, Irene Marchesi, Valentina Bordoni, Forcales Sonia Vanina, F. Calvisi Diego, Luigi BagellaAbstractRhabdomyosarcoma (RMS) is a pediatric tumor, which arises from muscle precursor cells. Recently, it has been demonstrated that Hippo Pathway (Hpo), a pathway that regulates several physiological and biological features, is involved in RMS tumorigenesis. For instance, an upregulation of the Hpo downstream effector Yes-Associated Protein 1 (YAP) leads to the development of embryonal rhabdomyosarcoma (eRMS) in murine activated muscle satellite cells. On the other hand, the YAP paralog transcriptional co-activator with PDZ-binding motif (TAZ) is overexpressed in alveolar rhabdomyosarcoma (aRMS) patients with poor survival.YAP and TAZ exhibit both cytoplasmic and nuclear functions. In the nucleus, YAP binds TEADs (TEA domain family members) factors and together they constitute a complex that is able either to activate the transcription of several genes such as MYC, Tbx5 and PAX8 or to maintain the stability of others like p73. Due to the key role of YAP and TAZ in cancer, the identification and/or development of new compounds able to block their activity might be an effective antineoplastic strategy. Verteporfin (VP) is a molecule able to stop the formation of YAP/TEAD complex in the nucleus.The aim of this study is to evaluate the action of VP on RMS cell l...
Source: Chemico Biological Interactions - Category: Biochemistry Source Type: research

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"Verteporfin exhibits anti-proliferative activity in embryonal and alveolar rhabdomyosarcoma cell lines". Chem Biol Interact. 2019 Sep 05;:108813 Authors: Sanna L, Piredda R, Marchesi I, Bordoni V, Sonia Vanina F, Diego FC, Bagella L Abstract Rhabdomyosarcoma (RMS) is a pediatric tumor, which arises from muscle precursor cells. Recently, it has been demonstrated that Hippo Pathway (Hpo), a pathway that regulates several physiological and biological features, is involved in RMS tumorigenesis. For instance, an upregulation of the Hpo downstream effector Yes-Associated Protein 1 (YAP) leads to ...
Source: Chemico-Biological Interactions - Category: Molecular Biology Authors: Tags: Chem Biol Interact Source Type: research
Abstract A natural isoquinoline alkaloid, berberine, has been known to exhibit anti-tumor activity in various cancer cells via inducing cell cycle arrest. However, it has not been investigated whether berberine and its analogs inhibit the growth of rhabdomyosarcoma (RMS), which is the most frequent soft tissue tumor in children. The present study examined the anti-tumor effects of berberine and palmatine on expansions of three human embryonal RMS cell lines; ERMS1, KYM1, and RD. Intracellular incorporation of berberine was relatively higher than that of palmatine in every RMS cell line. Berberine significantly inh...
Source: Bioscience, Biotechnology, and Biochemistry - Category: Biochemistry Authors: Tags: Biosci Biotechnol Biochem Source Type: research
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the pediatric cancer population. Survival among metastatic RMS patients has remained dismal yet unimproved for years. We previously identified t...
Source: Skeletal Muscle - Category: Research Authors: Tags: Research Source Type: research
ConclusionsThe data presented herein indicate that targeting FAK phosphorylation at distinct sites is a promising strategy in future treatment approaches for defined subgroups of rhabdomyosarcoma.
Source: Journal of Cancer Research and Clinical Oncology - Category: Cancer & Oncology Source Type: research
AbstractDICER1 syndrome is a rare tumor predisposition syndrome with manifestations that predominantly affect children and young adults. The syndrome is typically caused by heterozygous germline loss-of-functionDICER1 alterations accompanied on the other allele by somatic missense mutations occurring at one of a few mutation hotspots within the sequence encoding the RNase IIIb  domain.DICER1 encodes a member of the microRNA biogenesis machinery. The syndrome spectrum is highly pleiotropic and features a unique constellation of benign and malignant neoplastic and dysplastic lesions. Pleuropulmonary blastoma (PPB), the ...
Source: Acta Neuropathologica - Category: Neurology Source Type: research
ConclusionTo date, this is the largest study on pediatric sinonasal RMS. IRSG clinical risk groups may be useful in stratifying high ‐risk patients with poor prognosis.
Source: International Forum of Allergy and Rhinology - Category: Allergy & Immunology Authors: Tags: ORIGINAL ARTICLE Source Type: research
ConclusionsTaken together, our data suggest the strategic role of oxidant/antioxidant balance in restraining the therapeutic efficiency of RT in RMS treatment and identify NRF2 as a new potential molecular target whose inhibition might represent a novel radiosensitizing therapeutic strategy for RMS clinical management.
Source: Journal of Cancer Research and Clinical Oncology - Category: Cancer & Oncology Source Type: research
ConclusionTo date, this is the largest study on pediatric sinonasal RMS. IRSG clinical risk groups may be useful in stratifying high ‐risk patients with poor prognosis.
Source: International Forum of Allergy and Rhinology - Category: Allergy & Immunology Authors: Tags: ORIGINAL ARTICLE Source Type: research
Publication date: Available online 30 December 2018Source: Cancer GeneticsAuthor(s): Jianling Ji, Fariba Navid, Mathew C. Hiemenz, Maki Kaneko, Shengmei Zhou, Sulagna Saitta, Jaclyn A. BiegelAbstractGermline pathogenic variants in CBL are associated with an autosomal dominant RASopathy and an increased risk for malignancies, particularly juvenile myelomonocytic leukemia. Herein, we describe a patient with clinical features of a Noonan-spectrum disorder who developed embryonal rhabdomyosarcoma of the bladder at age two years. Tumor analysis using the OncoKids® cancer panel revealed a CBL pathogenic variant: NM_005188.3:...
Source: Cancer Genetics - Category: Cancer & Oncology Source Type: research
ConclusionsThis study suggests that the combined exposure to PARPi and IR might display a role in the treatment of RMS tumours compared with single-agent exposure, since stronger cytotoxic effects are induced, and compensatory survival mechanisms are prevented.
Source: Journal of Cancer Research and Clinical Oncology - Category: Cancer & Oncology Source Type: research
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